Goat anti-Argininosuccinate synthetase 1 Antibody | MyBioSource MBS421531 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-Argininosuccinate synthetase 1 Antibody

catalog :

MBS421531

quantity :

0.1 mg

price :

300 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human, mouse, rat, dog, cow

application :

western blot, immunohistochemistry, immunocytochemistry, immunoprecipitation, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS421531

products type :

Antibody

products full name :

Goat anti-Argininosuccinate synthetase 1 Antibody

products short name :

Argininosuccinate synthetase 1

products name syn :

Goat Anti-Argininosuccinate synthetase 1, Biotinylated Antibody; Argininosuccinate synthetase 1; ASS; ASS1; CTLN1; citrulline--aspartate ligase; Argininosuccinate synthetase 1 antibody; ASS antibody; ASS1 antibody; CTLN1 antibody; citrulline--aspartate ligase antibody; Argininosuccinate synthetase 1; Argininosuccinate synthetase 1

other names :

argininosuccinate synthase; Argininosuccinate synthase; argininosuccinate synthase; argininosuccinate synthase 1; Citrulline--aspartate ligase

products gene name :

ASS1

other gene names :

ASS1; ASS1; ASS; CTLN1; ASS

uniprot entry name :

ASSY_HUMAN

clonality :

Polyclonal

host :

Goat

reactivity :

Tested: Human, Cow; Expected from sequence similarity: Human, Mouse, Rat, Dog

sequence length :

412

sequence :

ENPKNQAPPGLYTKTQD

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA), Western Blot (WB), Immunofluorescence (IF), Immunoprecipitation (IP), Immunohistochemistry (IHC)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 64000. Immunohistochemistry: In paraffin embedded Human Kidney shows textured cytoplasm staining in PCT. Recommended concentration, 2-4ug/ml. Western Blot: Approx 45kDa band observed in Human Kidney lysates (calculated MW of 46.5kDa according to NP_000041.2 and NP_446464.1). Recommended concentration: 0.01-0.03ug/ml. Immunofluorescence: A customer has reported this antibody works well in IF. Immunoprecipitation: This antibody has been successfully used in IP from BAEC cells as described in the following paper: Corbin et al, Biochem Biophys Res Commun.2008 Dec 26;377(4): 1042-6, PMID: 18948083.

other info1 :

Immunogen: Peptide with sequence C-ENPKNQAPPGLYTKTQD, from the internal region of the protein sequence according to NP_000041.2 ; NP_446464.1. Epitope: Internal region

other info2 :

Note: The variants represent identical protein (NP_000041.2 and NP_446464.1).

ncbi gi num :

53759107

ncbi acc num :

NP_000041.2

ncbi gb acc num :

NM_000050.4

ncbi mol weight :

46,530 Da

ncbi pathways :

Alanine And Aspartate Metabolism Pathway (198783); Alanine, Aspartate And Glutamate Metabolism Pathway (101142); Alanine, Aspartate And Glutamate Metabolism Pathway (100063); Arginine Biosynthesis Pathway (82943); Arginine Biosynthesis Pathway (306); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); L-citrulline-nitric Oxide Cycle Pathway (703105); Metabolic Pathways (132956); Metabolism Pathway (1269956)

ncbi summary :

The protein encoded by this gene catalyzes the penultimate step of the arginine biosynthetic pathway. There are approximately 10 to 14 copies of this gene including the pseudogenes scattered across the human genome, among which the one located on chromosome 9 appears to be the only functional gene for argininosuccinate synthetase. Mutations in the chromosome 9 copy of this gene cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Aug 2012]

uniprot summary :

ASS1: Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1). Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or adulthood. Belongs to the argininosuccinate synthase family. Type 1 subfamily. Protein type: Amino Acid Metabolism - arginine and proline; Endoplasmic reticulum; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 6.3.4.5; Mitochondrial; Ligase. Chromosomal Location of Human Ortholog: 9q34.1. Cellular Component: cytoplasm; cytosol; endoplasmic reticulum; lysosome; mitochondrial outer membrane; nucleus; perikaryon. Molecular Function: amino acid binding; argininosuccinate synthase activity; ATP binding; identical protein binding; protein binding; toxin binding. Biological Process: acute-phase response; aging; arginine biosynthetic process; argininosuccinate metabolic process; aspartate metabolic process; citrulline metabolic process; kidney development; liver development; midgut development; positive regulation of nitric oxide biosynthetic process; response to drug; response to estradiol stimulus; response to mycotoxin; response to nutrient; response to zinc ion; urea cycle. Disease: Citrullinemia, Classic

size1 :

0.1 mg

price1 :

300 USD

more info or order :

MyBioSource product webpage